伴限局性Castleman病的副肿瘤性天疱疮临床及实验室研究

目的：报告6例伴限局性Castleman病副肿瘤性天疱疮，从临床、组织病理和免疫学方面进行了研究，从而能早期诊断，早期治疗。方法：采用全面临床检查，包括CT检查，常规组织病理检查，间接免疫荧光检查，免疫印迹及酶联免疫吸附测定（ELISA）。结果：6例患者临床上均有严重的口腔黏膜糜烂或溃疡。工有形态各异及程度不等的皮损，CT检查均示有潜在单发的内脏肿瘤。组织病理检查示表皮内疱，基底细胞液化变性，表皮内坏死角质形成细胞及真皮浅层以淋巴细胞为主的浸润。免疫学检查示患者血清中含抗棘细菌间抗体，它们能与桥粒的envoplakin及periplakin发生特异结合。手术切除后病理检查均为Castleman病，术后皮损逐渐消退。结论：副肿瘤性天疱疮无论在临床，皮损的组织病理学改变，还是免疫学上都具有特征性，对于临床上口腔黏膜有广泛糜烂或溃疡，按天疱疮治疗无效的患者，应考虑副肿瘤性天疱疮的可能性。早期诊断，及时切除肿瘤或作相应治疗，患者的预后是良好。

Clinical and experimental study of paraneoplastic pemphigus with Castleman's disease

Objective:This article reports6cases of paraneoplastic pemphigus(PNP)with Castleman's disease,including clin ical,histopathological and immunological study conducted,thus the disease can be diagnosed and treated in its early stage.Methods:Full sc`ale of physical examination,CT,histopathological and indirect immunofluorescent study,im munoblot and ELISA were com pleted.Results:Clinically all6cases showed severe erosion or ulcer of the oral mucosa with vari-form skin lesions.Solidary internal tumor was detected by CT in all cases.Histopathologically,there were in traepidermal a-can tholytic vesicle,basal cell liquefica tion,necrotic keratinocytes in the epidermis and lymphocyte infiltration in the upper dermis.Immunological exam showed IgG and C3deposit in the epidermal intercellular space which demonstrated there was au toantibody in the patient 's serum.Further study re vealed that these autoantibody specifically bound to envo plakin and periplakin of the desmosome.The tumor was removed by surgical incision and identified with Castleman's dis ease patho-logically.The mucosa and skin lesions were gradually went away after the removal of the tumor.Conclusions:PNP has characteristic features clinically,histopathologically as well as immunologically.The possibility of PNP can be con sidered if the patient manifests severe and extensive erosion and ulcer in the oral mucosa and does not response well to corticos-teroids.The prognosis of PNP will be good if it is diagnosed in the early stage and tumor removed and treated in a proper way.